Quantitative Relationships between Huntingtin Levels, Polyglutamine Length, Inclusion Body Formation, and Neuronal Death Provide Novel Insight into Huntington's Disease Molecular Pathogenesis

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¹Gladstone Institute of Neurological Disease,
²Taube-Koret Center for Huntington's Disease Research,
³Chemistry and Chemical Biology Program,
⁴Medical Scientist Training Program, and
⁵Neuroscience Program, University of California, San Francisco, California 94158,
⁶Biomedical Sciences Program, and
⁷Departments of Neurology and
⁸Physiology, University of California, San Francisco, California 94143,
⁹Department of Statistical Science, Duke University, Durham, North Carolina 27708, and
¹⁰Departments of Neurosciences and
¹¹Pathology, University of California, San Diego, La Jolla, California 92093

Correspondence should be addressed to Steven Finkbeiner, Gladstone Institute of Neurological Disease, 1650 Owens Street, San Francisco, California 94158. sfinkbeiner{at}gladstone.ucsf.edu

M. Arrasate's present address: Division of Neuroscience, Centro de Investigación Médica Aplicada, University of Navarra, Avenida Pío XII, 55, E-31008 Pamplona, Spain.

M. Arrasate's present address: Division of Neuroscience, Centro de Investigación Médica Aplicada, University of Navarra, Avenida Pío XII, 55, E-31008 Pamplona, Spain.

This work was supported by the National Institutes of Health (NIH)–National Institute of General Medical Sciences University of California, San Francisco (UCSF), Medical Scientist Training Program and a fellowship from the UCSF Hilblom Center for the Biology of Aging (J.M.). E.M. was supported, in part, by NIH Grants AG18440, AG11385, and AG022074. S.F. was supported by the Taube-Koret Center for Huntington's Disease Research, by NIH–National Institute of Neurological Disorders and Stroke Grants 2R01 NS039074 and 2R01 NS045091 and NIH–National Institute on Aging Grant 2P01 AG022074, and by The J. David Gladstone Institutes. The animal care facility was partly supported by an NIH Extramural Research Facilities Improvement Program Project (Grant C06 RR018928). We thank A. Kazantzev and D. Housman for the htt cDNAs and R. Tsien for the mRFP cDNA that we used to construct our mammalian expression plasmids. We thank members of the Finkbeiner Laboratory for useful discussions, G. Howard and S. Ordway for editorial assistance, and K. Nelson for administrative assistance.
Correspondence should be addressed to Steven Finkbeiner, Gladstone Institute of Neurological Disease, 1650 Owens Street, San Francisco, California 94158. sfinkbeiner{at}gladstone.ucsf.edu

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